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Biomedical subjects

F K Assadi

Publications and source records attributed to F K Assadi.

At least 19 recordsLinked to original sources

Poland syndrome associated with renal agenesis.

Poland syndrome is characterized by unilateral aplasia or hypoplasia of the sternocostal portion of the pectoralis major muscle and ipsilateral syndactyly. In some cases other associated anomalies, including renal malformations, dextrocardia, and vertebral abnormalities, have been reported. We report a 7-month-old girl with Poland syndrome who also presented with ipsilateral renal agenesis. This report suggests that renal structural anomaly may be an integral part of this syndrome. We recommend renal imaging studies be performed on all children with Poland syndrome.

Female↗

Hypercalciuria and urolithiasis in a case of Costello syndrome.

Costello syndrome is characterized by postnatal growth deficiency, mental retardation, curly hair, coarse characteristic face, and loose skin of hands and feet. Patients with this syndrome have a high incidence of cardiac involvement, including arrhythmia, atrial septal defect, and hypertrophic cardiomyopathy. We report a 16-year-old adolescent female with Costello syndrome who presents with hypercalciuria and urolithiasis.

Abnormalities, Multiple↗

Effects of pre- and postnatal cysteamine exposure on renal function in the rat.

The safety of cysteamine after renal transplantation and during pregnancy is an important issue, since girls with cystinosis are in better health on cysteamine therapy and thus more likely to become pregnant. In the first study, cysteamine was given to pregnant rats on days 6.5-18.5 post conception in oral doses of 0, 37.5, 75, 100, and 150 mg/kg per day. The dams were sacrificed on day 20.5, the fetal kidneys removed and prepared for histological examination. In the second study, cysteamine was given to dams on days 6.5-19.5 post conception in oral doses of 0, 37.5, 50, and 75 mg/kg per day. Dams were allowed to give birth naturally and pups were given cysteamine on days 4-21 to yield the same oral doses of cysteamine given to the dam. Renal function was evaluated on day 35. Histological examination of fetal kidneys revealed no changes even in kidneys from fetuses with growth retardation and malformations. Furthermore, there were no alterations in renal function in offspring on day 35. These findings demonstrate that cysteamine therapy does not affect renal development in the rat. Further investigations will be required to prove whether cysteamine therapy has the potential to affect renal development in the human.

Animals↗

Angiotensin converting enzyme inhibitor-induced angioedema: a report of two cases.

Angioedema is a rare but potentially fatal side effect of angiotensin converting enzyme (ACE) inhibitors. We report for the first time, two children with systemic lupus erythematosus who developed acute angioedema after the long-term use of enalapril. Prompt recognition and appropriate management of ACE-induced angioedema prevented life-threatening complications. This report highlights the potential risks of angioedema associated with the use of ACE inhibitors in children. Patients should be advised to seek medical treatment immediately if they experience swelling of the face, neck, or tongue, and especially if they have trouble breathing, speaking, or swallowing.

Adolescent↗

Coadministration of theophylline enhances diuretic response to furosemide in infants during extracorporeal membrane oxygenation: a randomized controlled pilot study.

OBJECTIVE: This pilot study evaluates the efficacy of low-dose theophylline administered before furosemide to enhance diuresis in neonates recovering from fluid retention during extracorporeal membrane oxygenation (ECMO). STUDY DESIGN: Infants receiving ECMO (n = 24) were randomized (double blind, placebo-controlled) to receive either regimen A (placebo/furosemide day 1, theophylline/furosemide day 2, placebo/furosemide day 3) or regimen B (theophylline/furosemide day 1, placebo/furosemide day 2, theophylline/furosemide day 3). Urine flows and renal functions were compared. RESULTS: Urine flow rate before initiation of diuretic therapy was not significantly different between groups A and B (2.6 +/- 1.4 vs 3.5 +/- 1.3 ml/kg/hr, respectively, p = 0.12). Infants who received theophylline/furosemide had significantly higher urine flow rates than those who received placebo/furosemide on day 1 (11.8 +/- 4.6 vs 7.2 +/- 2.4 ml/kg/hr, p < 0.01). The 24-hour fluid volumes and balances became significantly more negative with theophylline enhancement of furosemide's effect. There were no significant differences in renal function between the two groups. CONCLUSION: Low doses of theophylline given before furosemide administration significantly enhance diuretic response in infants with fluid retention during ECMO.

Diuresis↗

Urinary beta 2-microglobulin as a marker for vesicoureteral reflux.

To determine whether urinary beta 2-microglobulin (beta 2 M) excretion would be elevated in patients with severity of vesicoureteral reflux, urinary beta 2 M/creatinine (Cr) ratios were measured on random urine samples in 56 children with various grades of reflux. Results were compared with ratios of 39 nonrefluxing patients matched for age and gender. Patients with evidence of renal insufficiency or urinary tract infection were excluded. Bladder urine was obtained at the time of the vesicoureterogram. Reflux was graded using the International Reflux Classification System. The mean urinary beta 2 M/Cr ratio was higher in the refluxing group (1.82 +/- 0.6 micrograms/mg Cr) than in the nonrefluxing control group (0.54 +/- 0.09 microgram/mg Cr. P < 0.01). When the mean urinary beta 2 M/Cr ratios were compared for each grade of reflux with the nonrefluxing control group, patients with grade IV and V reflux had significantly higher urinary beta 2 M/Cr values than the nonrefluxing patients (2.83 +/- 0.71 micrograms/mg Cr and 4.61 +/- 0.65 micrograms/mg Cr, P < 0.001, respectively). No patient with grade I, II, or III reflux had a urinary beta 2 M/Cr ratio above 0.92 microgram/mg Cr. Statistical analysis revealed no significant differences among the mean beta 2 M/Cr ratio for grade I (0.53 +/- 0.08), II (0.51 +/- 0.09), or III (0.59 +/- 0.17) refluxers or the nonrefluxing controls. Therefore, urinary beta 2 M/Cr ratios are increased in children with a high grade of reflux. Such values may be useful in the early detection of tubular damage in patients with vesicoureteral reflux.

Child↗

Renal tubular dysfunction in fetal alcohol syndrome.

Renal function was evaluated in six patients with fetal alcohol syndrome (FAS) and eight control subjects before and after fluid restriction and acute acid loading. Baseline serum electrolytes, creatinine clearance, fractional sodium excretion, tubular reabsorption of phosphate, urine and blood pH and osmolalities, plasma renin activity, and plasma aldosterone level were normal in all subjects, but fractional potassium excretion (FEK) was lower in FAS patients than in control subjects (P less than 0.001). Despite equivalent plasma osmolalities (295 +/- 3 vs 293 +/- 2 mosmol/kg, P = 0.2), the maximum urinary osmolality after 12 h of water deprivation in patients with FAS was significantly lower compared with controls (560 +/- 107 vs 965 +/- 77 mosmol/kg; P less than 0.001) and increased to only 578 +/- 101 mosmol/kg after vasopressin administration. After ammonium chloride loading, minimum urine pH was significantly higher in patients than in controls (5.7 +/- 0.17 vs 4.81 +/- 0.19; P less than 0.001). Net acid excretion and FEK were also lower in patients than in controls (102 +/- 11 vs 139.6 +/- 11.3 microEq/min per 1.73 m2 and 23.5 +/- 1.3 vs 29 +/- 1.6%, respectively; P less than 0.001). The data indicate a subclinical renal tubular defect in urine concentration and acidification in patients with FAS.

Female↗

Acute hypertensive encephalopathy in minimal change nephrotic syndrome.

A 3-year-old child with minimal change nephrotic syndrome (MCNS) developed an acute hypertensive encephalopathy characterized by coma, focal seizures, right hemiparesis, global aphasia and cortical blindness. Episodic hypertension and seizures persisted for 24 h despite intervention with antihypertensive and anticonvulsant therapy. Clinical suspicion of cortical blindness was confirmed by visual-evoked potential studies. CT scans performed 14 and 21 days after the acute episode demonstrated symmetric occipital white matter lucencies compatible with ischemia and/or associated edema. Hypertensive encephalopathy with cortical blindness and symmetric white matter hypodense lesions visualized on CT scan have recently also been described in eclampsia of pregnancy. This report documents an unusual acute hypertensive encephalopathy in childhood MCNS, unassociated with membranoproliferative glomerulonephritis, or progressive focal glomerulosclerosis.

Blindness↗

Recurrent hypertonic dehydration due to selective defect in the osmoregulation of thirst.

A 6-year-old girl with recurrent episodes of hypertonic dehydration was studied. She denied thirst even with a plasma osmolality as high as 421 mosmol/kg. The hypernatremia was associated with an ability to concentrate urine (854 mosmol/kg). Volume expansion with water corrected hypernatremia (162 to 148 mEq/l) and resulted in an increased urine flow and urinary dilution (137 mosmol/kg) because of suppression of endogenous vasopressin (AVP) release (5.1 pg/ml). Hypertonic saline infusion raised the plasma AVP level (25.6 pg/ml) in response to changes in plasma osmolality (305 to 330 mosmol/kg) and led to a maximal urine osmolality of 818 mosmol/kg. With chronic forced fluid intake, the patient maintained a normal serum sodium concentration (range, 135-145 mEq/l) with a urine osmolality as low as 65 mosmol/kg. These findings are consistent with an isolated defect in the osmoregulation of thirst as the cause of the chronic hypertonic dehydration without deficiency in AVP secretion.

Arginine Vasopressin↗

Therapy of acute bronchospasm. Complicated by lactic acidosis and hypokalemia.

Hypokalemia and lactic acidosis developed following correction of respiratory acidosis in a 5-year-old child who presented with respiratory failure secondary to severe asthma and treated with theophylline, inhaled albuterol, and parenteral methylprednisolone. Calculation of the "anion gap" that provided the clue to presence of lactic acidosis was confirmed by the measurement of serum lactate level.

Acidosis, Lactic↗

Treatment of acute renal failure in an infant by continuous arteriovenous hemodialysis.

Continuous arteriovenous hemodialysis (CAVHD) was performed in a critically ill oliguric infant with progressive uremia using a miniature Amicon hemofilter. Modification was made in the filter system by circulating 2.5% Dianeal peritoneal dialysis fluid into the second port of the ultrafiltrate compartment to enable the filter to function by dialysis too (CAVHD). In comparison with continuous arteriovenous hemofiltration (CAVH), CAVHD provided superior urea clearance and adequate fluid removal, allowing the simultaneous administration of parenteral nutrition. The higher solute clearances in CAVHD make the technique superior to CAVH for renal replacement therapy in critically ill infants.

Acute Kidney Injury↗

Validity of whole blood osmolality measurement in sick neonates.

A comparison of heparinized whole blood with plasma osmolality measurements was performed on 100 sick newborns using a vapor pressure osometer. Aliquots of blood samples which had been collected into 1-ml syringes containing sodium heparin for blood gas analyses were used for osmolality determinations. There was excellent correlation between the two methods (r = 0.993). A total of 85 samples agreed within 2.0 units. In 38 samples the results obtained by the two methods were identical. The maximum observed discrepancy, present in only 2 samples, was 4 mosmol. Thus, the measurement of whole blood or plasma osmolality provides comparable information. The simplicity, the small volume of the blood sample required and the rapidity of the method make the determination of whole blood osmolality particularly useful in newborns requiring intensive care.

Blood Chemical Analysis↗

Renal handling of beta-2-microglobulin in neonates treated with gentamicin.

Increased levels of urinary beta 2-microglobulin (beta 2M) have been used as a marker of proximal tubular dysfunction in human neonates. To assess the value of beta 2M in the detection of early stages of tubular damage caused by gentamicin, renal handling of beta 2M was studied sequentially in 18 gentamicin-treated neonates with idiopathic respiratory distress syndrome (mean birth weight 1,781 g, mean gestational age 33.7 weeks) during the first 7 days of life. These data were compared with those obtained from 10 control infants matched for gestational and postnatal ages. In addition, follow-up studies of renal function were conducted in 14 of 18 study infants 1 week after termination of therapy, on day 14 postpartum. The (+/- SD) fractional tubular excretion of beta 2M (FE beta 2M) tended to decrease significantly in the control infants from 10.3 +/- 1% on day 1 to 6.5 +/- 0.8% on day 7 postpartum (p less than 0.05). In infants treated with gentamicin, the mean FE beta 2M rose from 10.5 +/- 2% on day 1 to 17.1 +/- 1% on day 7 (p less than 0.01), followed by a decrease to 8.2 +/- 0.5% over the next 7 days (p less than 0.001). Compared with the control infants, values for the infants receiving gentamicin were significantly higher on postpartum days 3,5, and 7 (p less than 0.001). No significant differences in serum creatinine, creatinine clearance, or fractional tubular excretion of sodium were observed between the two groups during the study period.(ABSTRACT TRUNCATED AT 250 WORDS)

Gentamicins↗